Pathology (Cardiovascular)

Question 1

What are the 3 main types and 1 less main type of cardiomyopathy?

Answer 1

Restrictive, dilated and hypertrophic. Other is arrhythmogenic right ventricular dysplasia.

Question 2

Describe what the heart looks like in dilated cardiomyopathy.

Answer 2

2 or 3 times normal size, flabby or floppy.

Question 3

What are the histology features of dilated cardiomyopathy?

Answer 3

Non-specific.

Question 4

What types of genetic mutation causes dilated cardiomyopathy?

Answer 4

Autosomal dominant, autosomal recessive, X-linked, mitochondrial.

Question 5

The genes that encode for what proteins are mutated in dilated cardiomyopathy.

Answer 5

Heart muscle proteins like desmin, dytrophin (muscular dystrophy).

Question 6

What are the other causes of dilated cardiomyopathy?

Answer 6

Toxins, alcohol (unknown if direct ethanol toxicity of nutritional deficiencies), doxorubicin (chemotherapy agent, assess heart prior to commencing chemotherapy).

Question 7

What are 2 rare causes of dilated cardiomyopathy?

Answer 7

Cardiac infection and pregnancy.

Question 8

What are the clinical features of dilated cardiomyopathy?

Answer 8

General picture of heart failure: SOB, poor exercise tolerance, low ejection fraction.

Question 9

Describe what the heart looks like in hypertrophic cardiomyopathy?

Answer 9

Big solid hearts, hypertrophic.

Question 10

What causes diastolic dysfunction in hypertrophic cardiomyopathy?

Answer 10

Heart cannot relax and eventually outflow obstruction due to left ventricular wall enlarging?

Question 11

What is hypertrophic cardiomyopathy a cause of in athletes?

Answer 11

Sudden death.

Question 12

What genes may be mutated in hypertrophic cardiomyopathy?

Answer 12

Beta myosin heavy chain, myosin binding protein, alpha tropomyosin.

Question 13

What are the histological features of hypertrophic cardiomyopathy?

Answer 13

Disorganised myofibres.

Question 14

What type of dysfunction does restrictive cardiomyopathy have?

Answer 14

Diastolic dysfunction.

Question 15

Describe the heart in restrictive cardiomyopathy.

Answer 15

Stiff.

Question 16

What happens to the atria as a result of restrictive cardiomyopathy?

Answer 16

Dilatation as a result of back pressure.

Question 17

What are the causes of restrictive cardiomyopathy?

Answer 17

Deposition of something in the myocardium: metabolic byproducts e.g. iron, amyloid, sarcoid (granulomas), tumours, fibrosis following radiation.

Question 18

What is amyloid?

Answer 18

Abnormal deposition of an abnormal protein.

Question 19

What do the abnormal proteins in amyloid tend to form?

Answer 19

Beta pleated sheets.

Question 20

How would you find out the cause of a restrictive cardiomyopathy?

Answer 20

Biopsy.

Question 21

What are the classifications of amyloid?

Answer 21

1. AA (related to chronic diseases like rheumatoid).
2. AL (light chains, abnormal Ig).
3. Haemodyalysis associated (beta 2 microglobulin).
4. Diabetes.
5. Alzheimer’s

Question 22

Give an example of a type of amyloid that is isolated in the heart?

Answer 22

Senile cardiac amyloidosis.

Question 23

What is they histological appearance of amyloid?

Answer 23

Waxy pink material, stains positively for congo red, exhibits apple green birefringence.

Question 24

Where is amyloid often seen affecting at autopsy, and what does this cause in a living person?

Answer 24

The conduction pathway. Arrhythmogenic death.

Question 25

What is arrhythmogenic right ventricular dysplasia and why is it hard to detect?

Answer 25

Right ventricle gets largely replaced by fat. The right ventricle always looks a bit fatty.

Question 26

What is the type of genetic disease that gives you arrhythmogenic right ventricular dysplasia?

Answer 26

Autosomal dominant with low penetrance.

Question 27

Is infective or non-infective myocarditis more common?

Answer 27

Infective.

Question 28

What are the most common infections in myocarditis?

Answer 28

Most are viral (Coxsackie A and B, ECHO virus, many others), Chaga's disease (trypanosomiasis in S america), borrelia burgdorferi (lyme's disease), HIV.

Question 29

What does the heart look like in infective myocarditis?

Answer 29

Thickened beefy myocardium.

Question 30

What will you see on histology of infectious myocarditis?

Answer 30

Inflammatory infiltrates.

Question 31

What causes non-infectious myocarditis?

Answer 31

Immune mediated hypersensitivity reactions e.g. hypersensitivity to infection (rheumatic fever after strep throat), hypersensitivity to drugs (eosinophilic myocarditis), systemic lupus erythematosus (SLE).

Question 32

What does rheumatic fever do to the heart?

Answer 32

Classic mitral stenosis with thickening and fusion of valve leaflets, short thick chordae tendinae, myocardium patchily inflamed.

Question 33

What will you see on histology of rheumatic fever?

Answer 33

Aschoff bodies (collage fibres and fibrinoid collagen necrosis, rheumatic granuloma).

Question 34

What are the causes of pericariditis?

Answer 34

Infection, immune mediated (rheumatic fever), idiopathic, uraemic (renal failure), post MI (Dressler's syndrome), connective tissue disease e.g. SLE.

Question 35

What kind of virus is usually in infectious pericarditis and what type of effusion does it cause?

Answer 35

ECHO virus, serous effusions.

Question 36

What kind of bacterial infections are there in pericarditis and what type of effusion do they produce?

Answer 36

Extension from elsewhere e.g. pnuemonia. Purulent effusions.

Question 37

What kind of patients get fungal pericarditis and what type of effusion do they produce?

Answer 37

Immunosuppressed patients, post-transplant, produce purulent effusions.

Question 38

What sort of material will be produced in the pericardial sac in tuberculous pericarditis?

Answer 38

Caseous material (cheesy).

Question 39

What is Dressler's syndrome?

Answer 39

Pericarditis post MI.

Question 40

How long after an MI does dresslers syndrome present?

Answer 40

Many weeks.

Question 41

What is assumed to be the cause of Dressler's syndrome?

Answer 41

Assumed to be immune mediated: damaged heart muscle releases previously unencountered material that stimulates an immune response.

Question 42

What are the complications of pericarditis?

Answer 42

Pericardial effusion, tamponade, constrictive pericarditis, cardiac failure, death.

Question 43

How virulent an organism causes infectious endocarditis?

Answer 43

Very virulent (may be bacterial or fungal).

Question 44

What historically caused infective endocarditis?

Answer 44

Rheumatic heart disease.

Question 45

What nowadays causes infective endocarditis?

Answer 45

Prosthetic valves, congential defects, bicuspid valves, MV prolapse, calcific disease.

Question 46

What are the parts of the HACEK acronym for bacteria that cause infectious endocarditis?

Answer 46

Haemophilus, actinobacillus, cardiobacteria, eikenella, kingella.

Question 47

What type of infective endocarditis do IV drug users get?

Answer 47

Candida, staph aureus (on right side).

Question 48

What type of bacteria infects prosthetic valves?

Answer 48

Staph epidermidis.

Question 49

What causes vegetations on heart valves?

Answer 49

Bacteria excite acute inflammation, bacteria and inflammatory cell products digest into the valve leaflets.

Question 50

What are the cardiac complications of infective endocarditis?

Answer 50

Acute valvular incompetence, high output cardiac failure, abscess, fistula and pericarditis.

Question 51

What are all the systemic manifestations of infective endocarditis?

Answer 51

Osler's nodes, Janeway lesions, roth spots, splinter haemorrhages, septicaemia, systemic septic emboli (brain, kidney etc), mycotic aneurysms.

Question 52

What are the causes of non-infective endocarditis?

Answer 52

Rheumatic fever, SLE, non-bacterial thrombotic endocarditis (marantic endocarditis), carcinoid heart disease.

Question 53

What is the effect of non-bacterial thrombotic endocarditis (NBTE) on heart valves?

Answer 53

Non-invasive and don't destroy valves, small and multiple vegetations.

Question 54

What is NBTE associated with?

Answer 54

Cancer (marantic). Frequently mucinous adenocarcinomas. Also hypercoagulable states.

Question 55

What is endocarditis caused by lupus known as?

Answer 55

Libman-Sacks endocarditis.

Question 56

Describe Libman-Sacks endocarditis.

Answer 56

Small sterile emboli often under surfaces of valves or on chords.

Question 57

What are carcinoid tumours?

Answer 57

Neoplasms of neuroendocrine cells.

Question 58

Where can you find carcinoid tumours?

Answer 58

In any mucosa (common in GI tract and lung).

Question 59

What carcinoid tumours release?

Answer 59

Hormones.

Question 60

What is carcinoid syndrome?

Answer 60

Occurs when carcinoid tumour has spread to the liver.

Question 61

What causes the symptoms of carcinoid syndrome?

Answer 61

Excess 5HIAA, serotonin, bradykinin etc production by the tumour.

Question 62

What are the symptoms and complications of carcinoid syndrome?

Answer 62

Flushing; nausea, vomiting and diarrhoea; produces right sided cardiac valve disease; tricuspid and pulmonary insufficiency.

Question 63

Why are primary tumours of the heart very rare?

Answer 63

Cardiac muscle cells are end differentiated.

Question 64

What is the commonest primary tumour of the heart?

Answer 64

Atrial myxoma.

Question 65

What kind of secondary tumour affect the heart?

Answer 65

Metastatic malignant melanoma, direct invasions by carcinoma of lung, oesophagus.

Question 66

What type of tumour can cause ball/valve obstruction?

Answer 66

Atrial myxoma?

Question 67

What can atrial myxoma cause?

Answer 67

Tumour emboli, endocarditis, systemic fever and malaise.